{"id":7644,"date":"2022-04-06T16:24:25","date_gmt":"2022-04-06T14:24:25","guid":{"rendered":"https:\/\/www.taranis-nutrition.com\/?page_id=7644"},"modified":"2022-09-01T17:26:02","modified_gmt":"2022-09-01T15:26:02","slug":"your-illness","status":"publish","type":"page","link":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/","title":{"rendered":"Your disease"},"content":{"rendered":"\n\n    \n        <div class=\"gk-wrapper container  no-bg\">\n            \n\n<h2 class=\"wp-block-heading\">Brief biochemical reminder and consequences of the enzyme deficiency<\/h2>\n\n\n\n<p>Depending on the residual enzymatic activity of phenylalanine hydroxylase, phenylalanine tolerance varies:<\/p>\n\n\n\n<ul><li>When blood levels of phenylalanine, with a normal diet, remain above 20mg\/100ml (1200 \u03bcmol\/l), <strong>PKU is termed classical (or typical)<\/strong> PKU with zero enzyme activity and a tolerance usually in the range of 250-300mg phenylalanine\/day.<\/li><li>When blood levels are between 10 and 20mg\/100ml (600 to 1200 \u03bcmol\/l), <strong>ECP is termed atypical<\/strong>, with low residual enzyme activity, but allowing for increased tolerance (up to 600 to 1000mg phenylalanine\/day).<\/li><li>When blood levels are below 10mg\/100ml (600 \u03bcmol\/l), <strong>this is termed permanent moderate hyperphenylalaninaemia <\/strong>and there is usually no need for diet. Normoprotein intakes should be maintained.<\/li><\/ul>\n\n\n\n<p><strong>This classification has its limitations<\/strong>, however, as it is based on the blood level of phenylalanine in the normal, i.e. phenylalanine-rich diet.<\/p>\n\n\n\n<p>Thus, an exclusively breastfed child may initially be considered to have moderate hyperphenylalaninaemia (&lt;10 mg\/100ml, as breastmilk is lower in phenylalanine than artificial milk), whereas his or her blood phenylalanine concentration will increase beyond 10 mg\/100ml upon weaning from breastmilk, ultimately classifying him or her as atypical PKU.<\/p>\n\n\n\n<p>In addition, there may be some degree of immaturity of the enzyme phenylalanine hydroxylase at birth, and improvement in phenylalanine tolerance is still possible in the first years of life.<\/p>\n\n\n\n<div class=\"wp-block-columns are-vertically-aligned-center is-layout-flex wp-container-core-columns-is-layout-1 wp-block-columns-is-layout-flex\">\n<div class=\"wp-block-column is-vertically-aligned-center is-layout-flow wp-block-column-is-layout-flow\">\n<p>There may be <strong>other forms of ECP<\/strong>.<\/p>\n\n\n\n<p>Under normal conditions, this enzyme needs a cofactor (see general principles), BH4 (Tetrahydrobiopterin), to function properly. There may also be forms of ECP that are sensitive to BH4. In these rare cases, the addition of oral BH4 in the form of a drug increases the residual enzymatic activity, with the result that the diet can be significantly extended (improvement of phenylalanine tolerance), or even the intake of the amino acid mixture can be stopped completely.<\/p>\n\n\n\n<p>Sensitivity to BH4 occurs mainly in atypical PKU, and is exceptional in classical PKU. It is therefore necessary to &#8220;test for sensitivity&#8221; to ensure a positive response and potential drug treatment. In the case of sensitive phenylketonuria treatable by BH4, the treatment will be &#8220;medicated&#8221;; the commercial form of BH4 has existed since 2009 in France, it is called KUVAN and is reimbursed at 100% by the French Social Security, with a marketing authorisation (AMM) for children over 4 years of age and not recommended during pregnancy.&nbsp;<strong>The current discussion concerns the real interest of a treatment with BH4 when it only allows a modest extension of the diet.<\/strong><\/p>\n\n\n\n<p>Finally, there are genuine deficits in the synthesis of BH4, which are revealed by hyperphenylalaninemia at the time of neonatal screening. In this case, it is not strictly speaking a &#8220;true phenylketonuria&#8221; due to phenylalanine hydroxylase deficiency, but a deficiency of its cofactor, BH4, which also acts as a cofactor in other enzymatic reactions. The treatment and subsequent monitoring is therefore different and will not be discussed here.<\/p>\n\n\n\n<p>Let&#8217;s consider the case of a child on a hypoprotein diet, which corresponds to the general principles but which will be explained to you in detail by your health centre team, and let&#8217;s address various issues that may arise <strong>when you go home<\/strong>.<\/p>\n<\/div>\n\n\n\n<div class=\"wp-block-column is-vertically-aligned-center is-layout-flow wp-block-column-is-layout-flow\"><div class=\"wp-block-image\">\n<figure class=\"alignright size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"600\" height=\"806\" src=\"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png\" alt=\"\" class=\"wp-image-7456\" srcset=\"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png 600w, https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1-223x300.png 223w\" sizes=\"(max-width: 600px) 100vw, 600px\" \/><\/figure><\/div><\/div>\n<\/div>\n\n\n        <\/div>\n\n    \n\n\n\n<div style=\"height: 50px\"><\/div>\n\n\n\n\n            <div class=\"gk-wrapper-round\">\n            <div class=\"curved-div upper\">\n                <svg viewBox=\"0 0 1920 40\">\n                    <path fill=\"#164194\" fill-opacity=\"1\" d=\"M0.4,40C36.7,35.2,433.5,3.2,903.5,3.1C1373.4,3,1920.4,40.2,1920.4,40.2H0.4V40z\"><\/path>\n                <\/svg>\n            <\/div>\n            <div class=\"gk-wrapper  \">\n                                    <div class=\"container\">\n                        \n\n<h2 class=\"has-text-align-center wp-block-heading\">More information on Phenylketonuria<\/h2>\n\n\n\n\n<div style=\"height: 50px\"><\/div>\n\n\n\n<div class=\"wp-block-columns is-layout-flex wp-container-core-columns-is-layout-2 wp-block-columns-is-layout-flex\">\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\">\n\n<a class=\"gk-link-image\" href=\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/diet-for-your-child-ecp\/\" target=\"\">\n            <div class=\"image-wrapper\">\n            <img decoding=\"async\" src=\"https:\/\/www.taranis-nutrition.com\/wp-content\/themes\/taranis\/cache\/rubrique-assiette-regime-500x375-c-default.jpeg\" alt=\"Aciduries organiques - Le r\u00e9gime pour votre enfant\">\n        <\/div>\n        <div class=\"desc-wrapper\">\n        <span>Your dietary plan<\/span>\n        <div class=\"icon-wrapper\">\n            <i><\/i>\n        <\/div>\n    <\/div>    \n<\/a><\/div>\n\n\n\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\">\n\n<a class=\"gk-link-image\" href=\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-travels\/\" target=\"\">\n            <div class=\"image-wrapper\">\n            <img decoding=\"async\" src=\"https:\/\/www.taranis-nutrition.com\/wp-content\/themes\/taranis\/cache\/rubrique-deplacement-1-500x375-c-default.jpeg\" alt=\"\">\n        <\/div>\n        <div class=\"desc-wrapper\">\n        <span>Your travels<\/span>\n        <div class=\"icon-wrapper\">\n            <i><\/i>\n        <\/div>\n    <\/div>    \n<\/a><\/div>\n\n\n\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\">\n\n<a class=\"gk-link-image\" href=\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/ecp-women-your-desire-to-get-pregnant\/\" target=\"\">\n            <div class=\"image-wrapper\">\n            <img decoding=\"async\" src=\"https:\/\/www.taranis-nutrition.com\/wp-content\/themes\/taranis\/cache\/rubrique-grossesse-500x375-c-default.jpeg\" alt=\"\">\n        <\/div>\n        <div class=\"desc-wrapper\">\n        <span>The desire to get pregnant<\/span>\n        <div class=\"icon-wrapper\">\n            <i><\/i>\n        <\/div>\n    <\/div>    \n<\/a><\/div>\n\n\n\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\">\n\n<a class=\"gk-link-image\" href=\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/problems-that-may-arise\/\" target=\"\">\n            <div class=\"image-wrapper\">\n            <img decoding=\"async\" src=\"https:\/\/www.taranis-nutrition.com\/wp-content\/themes\/taranis\/cache\/rubrique-pb-1-500x375-c-default.jpeg\" alt=\"\">\n        <\/div>\n        <div class=\"desc-wrapper\">\n        <span>Managing complex situations<\/span>\n        <div class=\"icon-wrapper\">\n            <i><\/i>\n        <\/div>\n    <\/div>    \n<\/a><\/div>\n<\/div>\n\n\n                    <\/div>\n                            <\/div>\n        <\/div>\n    ","protected":false},"excerpt":{"rendered":"","protected":false},"author":1,"featured_media":0,"parent":7629,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"footnotes":""},"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v22.9 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Your disease - Taranis<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Your disease - Taranis\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/\" \/>\n<meta property=\"og:site_name\" content=\"Taranis\" \/>\n<meta property=\"article:modified_time\" content=\"2022-09-01T15:26:02+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"3 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/\",\"url\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/\",\"name\":\"Your disease - Taranis\",\"isPartOf\":{\"@id\":\"https:\/\/www.taranis-nutrition.com\/#website\"},\"primaryImageOfPage\":{\"@id\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#primaryimage\"},\"image\":{\"@id\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#primaryimage\"},\"thumbnailUrl\":\"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png\",\"datePublished\":\"2022-04-06T14:24:25+00:00\",\"dateModified\":\"2022-09-01T15:26:02+00:00\",\"breadcrumb\":{\"@id\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#breadcrumb\"},\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/\"]}]},{\"@type\":\"ImageObject\",\"inLanguage\":\"en-US\",\"@id\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#primaryimage\",\"url\":\"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png\",\"contentUrl\":\"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png\"},{\"@type\":\"BreadcrumbList\",\"@id\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#breadcrumb\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https:\/\/www.taranis-nutrition.com\/en\/\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Your diet\",\"item\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/\"},{\"@type\":\"ListItem\",\"position\":3,\"name\":\"Phenylketonuria\",\"item\":\"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/\"},{\"@type\":\"ListItem\",\"position\":4,\"name\":\"Your disease\"}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/www.taranis-nutrition.com\/#website\",\"url\":\"https:\/\/www.taranis-nutrition.com\/\",\"name\":\"Taranis\",\"description\":\"Taranis\",\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/www.taranis-nutrition.com\/?s={search_term_string}\"},\"query-input\":\"required name=search_term_string\"}],\"inLanguage\":\"en-US\"}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Your disease - Taranis","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/","og_locale":"en_US","og_type":"article","og_title":"Your disease - Taranis","og_url":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/","og_site_name":"Taranis","article_modified_time":"2022-09-01T15:26:02+00:00","og_image":[{"url":"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png"}],"twitter_card":"summary_large_image","twitter_misc":{"Est. reading time":"3 minutes"},"schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"WebPage","@id":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/","url":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/","name":"Your disease - Taranis","isPartOf":{"@id":"https:\/\/www.taranis-nutrition.com\/#website"},"primaryImageOfPage":{"@id":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#primaryimage"},"image":{"@id":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#primaryimage"},"thumbnailUrl":"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png","datePublished":"2022-04-06T14:24:25+00:00","dateModified":"2022-09-01T15:26:02+00:00","breadcrumb":{"@id":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#breadcrumb"},"inLanguage":"en-US","potentialAction":[{"@type":"ReadAction","target":["https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/"]}]},{"@type":"ImageObject","inLanguage":"en-US","@id":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#primaryimage","url":"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png","contentUrl":"https:\/\/www.taranis-nutrition.com\/wp-content\/uploads\/2022\/04\/Fig-7-schema-simplifie-du-metabolisme-de-la-phenylalanine-1-e1494506922616-600x806-1.png"},{"@type":"BreadcrumbList","@id":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/your-illness\/#breadcrumb","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https:\/\/www.taranis-nutrition.com\/en\/"},{"@type":"ListItem","position":2,"name":"Your diet","item":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/"},{"@type":"ListItem","position":3,"name":"Phenylketonuria","item":"https:\/\/www.taranis-nutrition.com\/en\/your-diet\/phenylketonuria\/"},{"@type":"ListItem","position":4,"name":"Your disease"}]},{"@type":"WebSite","@id":"https:\/\/www.taranis-nutrition.com\/#website","url":"https:\/\/www.taranis-nutrition.com\/","name":"Taranis","description":"Taranis","potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/www.taranis-nutrition.com\/?s={search_term_string}"},"query-input":"required name=search_term_string"}],"inLanguage":"en-US"}]}},"_links":{"self":[{"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/pages\/7644"}],"collection":[{"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/comments?post=7644"}],"version-history":[{"count":6,"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/pages\/7644\/revisions"}],"predecessor-version":[{"id":8783,"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/pages\/7644\/revisions\/8783"}],"up":[{"embeddable":true,"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/pages\/7629"}],"wp:attachment":[{"href":"https:\/\/www.taranis-nutrition.com\/en\/wp-json\/wp\/v2\/media?parent=7644"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}